The Hard Road - Part VI - The End of the Beginning

Before I begin, I will tell you the end of the story.

We don't know.

The doctors don't know.

Nobody knows.

But God knows.

How hard and how long the road will be for precious Joy. 

Between Wednesday night and Thursday we had four visits with the Boston pulmonary hypertension cardiologists and their nurse practitioner. Each of them spent well over 30 minutes with us and answered every question we could think of. Because we saw them individually we felt good about getting each of their personal opinions on a treatment plan for Joy. One of the doctors, Mary Mullen, had been consulted and already knew Joy on paper so it was nice to meet her, especially since we may land in her office down the road.

One thing we decided to do was an unofficial sleep study by trying Joy off oxygen this last night in the hospital where she could be monitored. Who knew but sleep apnea contributes to pulmonary hypertension. I was still awake around 11:30 when those oyygen numbers took a nosedive and the heart rate jumped. Less than one minute later the nurse was in putting Joy's oxygen cannula back in. The nurse wasn't messing around with that at all. She had seen enough. Joy slept through the whole thing and stabilized after awhile.

By Thursday morning, Joy had only one small IV left. She amazed me by peddling the tricycle all the way around the loop at the hospital. We are still discovering what she can and can't do. This was a very nice surprise as her gross motor skills are a bit behind.

We had to keep the IV because in order to go home she had to give up some blood, go for another chest x-ray and then a lung scan. They injected some radioactive stuff and took pictures. Joy was a trooper and the medical staff sure appreciated her easy going nature.

Once the lung scan was done, we had a meeting with the PH doctor and his nurse practitioner together for about 45 minutes. While we were waiting, the clowns showed up to entertain us. 

All of the Boston doctors were great about not making us feel like we had to choose or change cardiologists based on what we had learned during our adventure here. They said we could use them as much or as little as we wanted. They seemed genuinely happy to help us in any way. They gave us their protocols for medications, opinions on home oxygen use, and a few recommendations for the future. 

After our meeting, the nurse took out the last IV, did our paperwork, and got us out of there. Last stop was a good bye visit with our friend Ivy Joy and a walk down the hall together.

We packed up, paid for parking, and headed out of town. 

Halfway home, we stopped at Panera for dinner where Joy saw her first snow. I wish I had caught a picture of her face. She was NOT impressed. 

I realized over the last two days in Boston that 24/7 oxygen was depressing Joy. She is so developmentally behind and keeping her tethered to a machine was not helping her expand her horizons as quickly as she wanted to. The Boston doctors could not definitely tell us if the oxygen was necessary. We know it helps therapuetically but I questioned the need if her saturation levels were at 98% while she was sitting around the house. Once we followed up with our own cardiologist, we agreed to try Joy on oxygen only at night and naps for now and promised to keep track of the frequency of the "blue spells" that you can see in the picture above. 

Her delightful personality is back en force and Joy is loving life again with sugar and spice. She had a big language jump while we were in Boston and we were delighted to find out that she knows quite a few more words than we realized. She has added "thank you", "yummy", and "night-night" to her list which also includes potty, hello, bye-bye, and kitty. Of course, if you added all of our names, the list doubles.

We met with our cardiologist two weeks later and immediately scheduled a sleep study. Fortunately, she rolled with it beautifully. She was pretty used to the wires and the cannula so she fell right asleep. Looks like our girl has some moderate sleep apnea. We are waiting to find out what's next in that department. We also saw the endocrinologist this week and are scheduled to see a geneticist very soon. Because of this new development, I don't think we can claim "stable" quite yet but Joy is definitely holding her own for the time being. We will continue to tweak her meds and she will have another cath sometime in the next 7-9 months. We know that we added some years to Joy's life by inserting the occluder but nobody knows how many or is even willing to guess.

Thank you for being part of our journey with Joy, for the prayers on her behalf and the support of our family. We are grateful to be together as a family with children who are enjoying an excellent quality of life.

These truly are the best days of my life!